18F-FDG PET/CT features and management of primary cardiac lymphoma: a case report : Cardiology Plus

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18F-FDG PET/CT features and management of primary cardiac lymphoma: a case report

Wang, Ming1,2; Zhang, Shuyi1,2; Han, Wenzheng1,2,*

Author Information
Cardiology Plus 7(4):p 205-209, October-December 2022. | DOI: 10.1097/CP9.0000000000000027
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Abstract

BACKGROUND

Primary cardiac lymphoma (PCL) is a rare form of non-Hodgkin lymphomas. Diffuse large B-cell lymphoma (DLBCL) is the most common histologic type. Patients with PCL manifest symptoms and signs of heart and/or pericardium involvement with no extracardiac involvement[1]. Specific clinical presentation is variable and depends on the site of the lesion. Without treatment, PCL is rapidly fatal. Here, we an 81-year-old man, initially receiving surgical resection for a preliminary diagnosis of myxoma, but with pathologically proven DLBCL. The patient received three cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) but no subsequent anti-tumor therapy. At the last follow-up 3.5 years later, he was still alive.

CASE PRESENTATION

An 81-year-old man presented with progressive heart failure (eg, shortness of breath and palpitations) for 2 months (Table 1). The body weight had increased for about 5 kg during the past 2 months. He had hypertension and type 2 diabetes. There was no chest pain, fever, cough, abdominal pain, or nausea. Body temperature was normal. Physical examination revealed bilateral rales and wheezes, serious pitting edema in the lower legs, and bradycardia. No lymphadenopathy was noticed. The study was conducted in accordance with the Declaration of Helsinki (as revised in 2013) and approved by the committee board of Huadong Hospital affiliated to Fudan University. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written informed consent is available for review by the editorial office of this journal.

Table 1 - Timeline
Time Events
Two months before presentation Symptoms and signs of heart failure, including dyspnea and lower limb edema
First day at the emergency department Progressively aggravated shortness of breath
First day in heart failure unit ECG showed atrial flutter. ECHO revealed a large mass in the right atrium
Two weeks in heart failure unit PET-CT showed nodular uptakes in tumor lesions in the right atrium (SUVmax, 21.7)
A thoracotomy was conducted to remove the mass in the right atrium. Pathology revealed PCL
Four weeks in hematology department He developed multiple episodes of severe arrhythmia, tamponade, shock, and heart failure during R-CHOP treatment, but survived
3.5 years follow-up Occasional symptoms and signs of heart failure
ECG: Electrocardiogram; ECHO: Echocardiogram; PCL: Primary cardiac lymphoma; PET/CT: Positron emission tomography-computed tomography; SUVmax: High maximum standard uptake values; R-CHOP: Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.

Diagnosis

Electrocardiogram showed atrial flutter with 2:1 atrioventricular block (Figure 1A). Normalization to sinus rhythm after treatment with chemotherapy and surgery (Figure 1B). Echocardiogram revealed a 31 × 55 mm mass with unclear boundary in the right atrium (Figure 1C). The mass was connected to the atrial septum and extended to the tricuspid valves and the superior vena cava. Moderate tricuspid regurgitation was noticed. Systolic pulmonary artery pressure was 51 mmHg. Tricuspid annular plane systolic excursion (TAPSE) was 12 mm. LVEF was 55%. A preliminary diagnosis of cardiac myxoma was made. 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) confirmed a mass in the right atrium. PET-CT also revealed non-homogeneous thickening of the right atrial and increased high maximum standard uptake values (SUVmax) of 18F-FDG throughout the mass (Figure 2A, B).

F1
Figure 1.:
12-Lead electrocardiogram and transthoracic echocardiogram upon presentation. A, The electrocardiogram before treatment. B, The electrocardiogram after treatment. C, Echocardiogram revealed a 31 × 55 mm mass in the right atrium.
F2
Figure 2.:
18F-FDG PET/CT and immunohistochemistry of PCL. A and B, MIP and axial image showed nodular uptakes in tumor lesions in the right atrium (SUVmax, 21.7). C, H&E staining showed clusters of atypical large B lymphoid cells. D, E, F, Immunostaining with CD20, Ki-67, and LCA. Magnification: ×100. 18F-FDG PET/CT: 18F-fluorodeoxyglucose positron emission tomography-computed tomography; AO: Aorta; DA: Descending aorta; LA: Left atrium; LV: Left ventricle; PCL: Primary cardiac lymphoma; RA: Right atrium; RVOT: Right ventricle outflow tract.

Thoracotomy was conducted to resect the mass in the right atrium. Gross inspection revealed a 6 × 6 cm lobulated, yellowish white mass with soft texture and unclear boundary. The mass originated in the superior vena cava and invaded the right atrial wall. Pathologic examination of the resected specimen revealed clusters of atypical large B lymphoid cells. Immunohistochemical staining revealed characteristics consistent with large B-cell lymphoma (type GCB, II, IPI score: 4). The tumor cells were positive staining for CD20, LCA, CD79a, BCL-6, BCL-2, negative staining for Pax5, CD3, CD5, CD10, CD30, CD15, ALK, CYCLIND1, CD21, CD23, EMA, SOX10, S-100 and exhibited a high proliferation index as illustrated by Ki-67 staining: 80% positive (Figure 2C–F).

Treatment

Four weeks after surgery, the patient started receiving and completed 3 cycles of R-CHOP regimen. He experienced multiple episodes of the fatal arrhythmia, tamponade, shock, and heart failure, but survived and was eventually discharged. He received no subsequent anti-tumor treatment. At the last follow-up after 3.5 years, he was still alive.

DISCUSSION

The index patient had progressive heart failure. Based on the results of echocardiogram, surgery was conducted to remove the mass in the right atrium. Pathologic examination of the resected specimen, however, revealed large B-cell lymphoma. Since there was no extracardiac involvement, the final diagnosis was PCL, and the patient received three cycles of R-CHOP regimen.

PCL accounts for approximate 0.5% of all extra nodal lymphomas and 1.3% of all cardiac tumors[2]. PCL typically occurs in elderly women (median age of 64 on disease onset)[1,3]. In majority of the cases, PCL occurs in immunocompromised individuals. The most frequent site includes the right atrium, and right ventricle, but may occasionally occur in major vessels (eg, pulmonary artery, superior vena cava, inferior vena cava, and the valves)[4]. However, our case was an elderly man with immunocompetent and negative of the HIV test. Symptoms and signs vary substantially based on the location, but most frequently include dyspnea, chest pain, and syncope. Other cardiac manifestations include arrhythmia, atrioventricular block, atrial fibrillation, supraventricular tachycardia, right bundle branch block, sinus bradycardia, ventricular tachycardia, and ventricular fibrillation[4]. Extracardiac manifestations include fever and weight loss[5].

Diagnosis of PCL is challenging since the absence of extracardiac involvement and non-specific clinical features. A comprehensive approach that includes echocardiogram, CT scan, MRI, and PET-CT is needed. Localized, heterogeneous elevated uptake of 18F-FDG in PET-CT tends to suggest malignancy, but again, is non-specific for PCL[6–8]. Serological inflammatory markers for sarcoidosis, particularly soluble IL-2 receptor (sIL-2R) and neopterin, are useful. Histologic examination using biopsy or surgical samples is required to confirm the diagnosis[9].

PCL is rapidly fatal if untreated. With appropriate treatment, the median overall survival is 4–5 years[1,10]. There has been no guidelines for the management of PCL. R-CHOP is the most widely treatment since this regimen has been shown to prolong progression-free survival in patient with B-cell lymphoma[11]. The use of doxorubicin in R-CHOP is associated with a variety of severe side effects, most notably, dose-dependent irreversible cardiotoxicity[12]. Surgery is indicated in patients with obstructed blood flow, but is unlikely to alters the long-term prognosis[2]. We conducted an electronic search to summarize the clinical characteristics and treatment patterns of PCL and identified 25 cases in China. The results are shown in Table 2.

Table 2 - Summary of reported cases studies with PCL published in China
No. Authors Published year Age (year) Sex Presentation Lymphoma classification Location Diagnostic method Treatment Outcome PMID
PET-CT CMR Surgery Chemotherapy
1 Chao TY, et al 1995 57 Male Dyspnea DLBCL LA, RA + Biopsy + 7571977
2 Wu ZR, et al 2006 70 Female Dyspnea and edema DLBCL RA + Partial 17062357
resection
3 Chen XZ, et al 2010 54 Male Edema DLBCL RA + Partial + Survival after 3 years follow-up 20666276
resection
4 Yu L, et al 2011 27 Male Edema and cough DLBCL RA + Partial + Survival after 3 years follow-up 22199453
resection
5 Chen KW, et al 2012 36 Male Dyspnea DLBCL RA Endomyocardial biopsy + Survival after 6 months follow-up 22808929
6 Liu DW, et al 2012 41 Female Swelling DLBCL RA Partial Died 6 months after surgery 22581865
resection
7 Yang JY, et al 2013 53 Male Dyspnea DLBCL RA + Heart transplantation Survival after 12 months follow-up 24297716
8 Liu XY, et al 2015 52 Male Body numbness DLBCL RA Total resection Survival after 5 months follow-up 25973119
9 Liu Y, et al 2015 61 Female Dyspnea DLBCL RA + Partial Died two weeks after surgery 26590890
resection
74 Male Dyspnea DLBCL RA + Biopsy Died one week after biopsy
10 Hu S, et al 2017 73 Male Dyspnea DLBCL RA + Partial + Survival after 6 months follow-up 28836560
resection
27 Female Palpitation DLBCL RA + + Partial + Survival after 20 months follow-up
resection
11 Wan Y, et al 2017 65 Female Facial puffiness DLBCL RA + Total resection + Survival after 11 months follow-up 28985492
12 Xiao YY, et al 2017 64 Female facial puffiness DLBCL RA + Partial 27807049
resection
13 Gong GH, et al 2018 40 Male Dyspnea DLBCL RA Partial 31949636
resection
14 Wang SY, et al 2018 55 Male Palpitation DLBCL RA, RV + + Partial + Survival after 12 months follow-up 30315591
resection
15 Li YT, et al 2019 48 Male Syncope DLBCL RA Partial + Died 4months after surgery 31141190
resection
16 Liu S, et al 2019 46 Male Dyspnea DLBCL RA + Partial + Survival after 12 months follow-up 31237548
resection
17 Wei X, et al 2019 51 Male Palpitation TCL RA Partial + Died 2 days after surgery 31280315
resection
18 Zhang JJ, et al 2019 57 Male Dyspnea DLBCL RA Partial + Survival after 2 months follow-up 31806993
resection
19 Chen X, et al 2020 68 Male Chest tightness Burkitt’s lymphoma RA + + Percutaneous biopsy + Survival after 1 year follow-up 32782699
20 Yang X, et al 2020 40 Female Chest congestion NKTCL RA + Partial Died some days after the surgery 35117194
resection
21 Yu JF, et al 2021 71 Male Dyspnea DLBCL RV + + Trans nasal endoscopy 33529498
22 Chang L, et al 2022 77 Female Dyspnea DLBCL LA, RA + + Biopsy + Survival after chemotherapy 35184743
23 Chen PP, et al 2022 47 Female Palpitation T-Cell Lymphoma LA + Endomyocardial biopsy + 35184743
24 Hu BW, et al 2022 70 Male Dyspnea palpitation DLBCL RA + Percutaneous biopsy + Survival after 6 months follow-up 35387513
25 Yang DY, et al 2022 79 Female Chest pain DLBCL RA + Examination of pericardial effusion Died one year after diagnosis 35463758
CMR: Cardiovascular magnetic resonance; DLBCL: Diffuse large B-cell lymphoma; NKTCL: Natural killer/T-cell lymphoma; LA: Left atrium; PCL: Primary cardiac lymphoma; PET/CT: Positron emission tomography-computed tomography; RA: Right atrium, RV: Right ventricle.

CONCLUSION

PCL tends to be misdiagnosed until pathologic examination of tissue specimen. PCL should be suspected in patients with rapidly growing mass in the right atrium and elevated 18F-FDG uptake in PET-CT. Appropriate anti-tumor therapy (eg, R-CHOP) could improve prognosis.

FUNDING

This work was supported by Shanghai Key Laboratory of Clinical Geriatric Medicine (13dz2260700).

AUTHOR CONTRIBUTIONS

WM wrote the first draft. ZSY participated in collecting clinical data. HWZ revised the manuscript. All authors reviewed and approved the final manuscript.

CONFLICTS OF INTEREST STATEMENT

The authors declare that they have no conflict of interest with regard to the content of this manuscript.

DATA SHARING STATEMENT

All data generated or analyzed during this study are included in this published article.

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Keywords:

Primary cardiac lymphoma; Diffuse large B-cell lymphoma; Right atrium; R-CHOP

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